Hereditary Tyrosinemia Type 1 Mice under Continuous Nitisinone Treatment Display Remnants of an Uncorrected Liver Disease Phenotype
Neuckermans, Jessie, Lequeue, Sien, Claes, Paul, Heymans, Anja, Hughes, Juliette H, Colemonts-Vroninks, Haaike, Marcélis, Lionel, Casimir, Georges, Goyens, Philippe, Martens, Geert A, Gallagher, James A, Vanhaecke, Tamara, Bou-Gharios, George, De Kock, Joery
Published in Genes (01.03.2023)
Published in Genes (01.03.2023)
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Metabolomic studies in the inborn error of metabolism alkaptonuria reveal new biotransformations in tyrosine metabolism
Norman, Brendan P., Davison, Andrew S., Hughes, Juliette H., Sutherland, Hazel, Wilson, Peter JM, Berry, Neil G., Hughes, Andrew T., Milan, Anna M., Jarvis, Jonathan C., Roberts, Norman B., Ranganath, Lakshminarayan R., Bou-Gharios, George, Gallagher, James A.
Published in Genes & diseases (01.07.2022)
Published in Genes & diseases (01.07.2022)
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Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria
Norman, Brendan P, Davison, Andrew S, Hickton, Bryony, Ross, Gordon A, Milan, Anna M, Hughes, Andrew T, Wilson, Peter J. M, Sutherland, Hazel, Hughes, Juliette H, Roberts, Norman B, Bou-Gharios, George, Gallagher, James A, Ranganath, Lakshminarayan R
Published in Metabolites (29.09.2022)
Published in Metabolites (29.09.2022)
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Conditional targeting in mice reveals that hepatic homogentisate 1,2-dioxygenase activity is essential in reducing circulating homogentisic acid and for effective therapy in the genetic disease alkaptonuria
Hughes, Juliette H, Liu, Ke, Plagge, Antonius, Wilson, Peter J M, Sutherland, Hazel, Norman, Brendan P, Hughes, Andrew T, Keenan, Craig M, Milan, Anna M, Sakai, Takao, Ranganath, Lakshminarayan R, Gallagher, James A, Bou-Gharios, George
Published in Human molecular genetics (01.12.2019)
Published in Human molecular genetics (01.12.2019)
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Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria
Hughes, Juliette H., Wilson, Peter J. M., Sutherland, Hazel, Judd, Shirley, Hughes, Andrew T., Milan, Anna M., Jarvis, Jonathan C., Bou‐Gharios, George, Ranganath, Lakshminarayan R., Gallagher, James A.
Published in Journal of inherited metabolic disease (01.03.2020)
Published in Journal of inherited metabolic disease (01.03.2020)
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Anatomical Distribution of Ochronotic Pigment in Alkaptonuric Mice is Associated with Calcified Cartilage Chondrocytes at Osteochondral Interfaces
Hughes, Juliette H., Keenan, Craig M., Sutherland, Hazel, Edwards, Henry R., Wilson, Peter J. M., Ranganath, Lakshminarayan R., Jarvis, Jonathan C., Bou-Gharios, George, Gallagher, James A.
Published in Calcified tissue international (01.02.2021)
Published in Calcified tissue international (01.02.2021)
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Journal Article
Homogentisic acid is not only eliminated by glomerular filtration and tubular secretion but also produced in the kidney in alkaptonuria
Ranganath, Lakshminarayan R., Milan, Anna M., Hughes, Andrew T., Khedr, Milad, Davison, Andrew S., Shweihdi, Ella, Norman, Brendan P., Hughes, Juliette H., Bygott, Helen, Luangrath, Emily, Fitzgerald, Richard, Psarelli, Eftychia E., Kan, Christa, Laan, Dinny, Olsson, Birgitta, Rudebeck, Mattias, Mankowitz, Louise, Sireau, Nicolas, Arnoux, Jean‐Baptiste, Le Quan Sang, Kim‐Hanh, Jarvis, Jonathan C., Genovese, Federica, Braconi, Daniela, Santucci, Annalisa, Zatkova, Andrea, Glasova, Helena, Stančík, Roman, Imrich, Richard, Rhodes, Nicholas P., Gallagher, James A.
Published in Journal of inherited metabolic disease (01.07.2020)
Published in Journal of inherited metabolic disease (01.07.2020)
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Efficacy and safety of once-daily nitisinone for patients with alkaptonuria (SONIA 2): an international, multicentre, open-label, randomised controlled trial
Ranganath, Lakshminarayan R, Psarelli, Eftychia Eirini, Arnoux, Jean-Baptiste, Braconi, Daniela, Briggs, Michael, Bröijersén, Anders, Loftus, Nadia, Bygott, Helen, Cox, Trevor F, Davison, Andrew S, Dillon, Jane P, Fisher, Michael, FitzGerald, Richard, Genovese, Federica, Glasova, Helena, Hall, Anthony K, Hughes, Andrew T, Hughes, Juliette H, Imrich, Richard, Jarvis, Jonathan C, Khedr, Milad, Laan, Dinny, Le Quan Sang, Kim-Hanh, Luangrath, Emily, Lukáčová, Ol'ga, Milan, Anna M, Mistry, Alpesh, Mlynáriková, Vanda, Norman, Brendan P, Olsson, Birgitta, Rhodes, Nicholas P, Rovenský, Jozef, Rudebeck, Mattias, Santucci, Annalisa, Shweihdi, Ella, Scott, Ciarán, Sedláková, Jana, Sireau, Nicolas, Stančík, Roman, Szamosi, Johan, Taylor, Sophie, van Kan, Christa, Vinjamuri, Sobhan, Vrtíková, Eva, Webb, Chris, West, Elizabeth, Záňová, Elizabeth, Zatkova, Andrea, Gallagher, James A
Published in The lancet. Diabetes & endocrinology (01.09.2020)
Published in The lancet. Diabetes & endocrinology (01.09.2020)
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Studies in alkaptonuria reveal new roles beyond drug clearance for phase I and II biotransformations in tyrosine metabolism
Norman, Brendan P, Davison, Andrew S, Hughes, Juliette H, Sutherland, Hazel, Wilson, Peter Jm, Berry, Neil G, Hughes, Andrew T, Milan, Anna M, Jarvis, Jonathan C, Roberts, Norman B, Ranganath, Lakshminarayan R, Bou-Gharios, George, Gallagher, James A
Published in bioRxiv (16.04.2020)
Published in bioRxiv (16.04.2020)
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